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Flashcards in Rheumatologic Lung DZ Deck (68)
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1

What are the five rheumatologic diseases that often present with pleuropulmonary symptoms

Systemic sclerosis, systemic lupus rheumatoid arthritis, dermatomyositis, Sjrogen Syndrome

2

Which of the fiv efeatures the MOST lung dz symptoms

Systemic sclerosis (aka scleroderma)

3

Lung dz in scleroderma takes two forms, they are:

Interstitial and Vascular

4

Interstitial lung dz in scleroderma or "SCLERODERMA LUNG" most often presesnts as

- non-specific interstitial pneumonia or interstitial fibrosis
- Concentric arterial thickening

5

About 67% of systemic sclerosis pts get

interstitial lung dz

6

About 20% of systemic sclerosis pts suffer from

pulmonary hypertension

7

The pathophysiological mediators in Scleroderma Lung are:

IL-8, TNF,

MIF1alpha

RANTES

Endothelin 1
TGF-Beta

8

IL8 does
TNF alpha does

IL8= neutrophil attractant and activator
TNFalpha-= neutrophil attractant and promoter of neutrophil endothelial binding

9

MIF1 alpha does

promoter of neutrophil chemotaxis

10

RANTES

T cell recruiter and activator

11

Endothelin 1

Vasoconstrictor

12

TGF-beta

promoter of fibroplasia

13

Scleroderma lung favors which part of the lung?

Base, posterior, periphery.

You will first see Ground glass appearance on the CT then will become more reticular

14

Early histology of scleroderma lung shows

Non-specific interstitial pneumonia with lymphocytes and macrophages followed by fibroblasts and collagen

15

Trichrome stain stains collagen what color?

blue

16

The vascular disease component of scleroderma lung causes

pulmonary hypertension and cor pulmonaale

17

What is CREST syndrome

When the the vascular component of scleroderma appears without the interstitial lung disease.
It is associated with anti-centromere antibodies and limited systemic sclerosis

18

What are the symptoms of SS lung dz

Dyspnea and dry cough due to increased work load of breathing due to fibrosis

19

Signs of SS Lung

Crackles due to the sudden opening of small airways abnormally closed by the pressure of interstitial inflammation

20

SS Lung dz diagnosis

Associtation of symptoms , signs, and radiology of interstitial lung dz with skin, esophageal, and renal manifestations of scleroderma.

- Anti-scl70 (DNA Topoisomerase) antibodies

21

Treatment of systemic sclerosis

Cyclophosphamide

22

Why don't you treat SS lung dz with steroids?

Renal crisis has been reported

23

Most common cause of death for scleroderma pts

Lung Disease

24

Prognosis of scleroderma pts from initial survival?

12 years

25

Pleuropulmonary disease of lupus generally has 5 manifestations. THey are:

- Pleuritis
- Acute Lupus pneumonitis
- Nonspecific interstitial pneumonia
- Pulmonary Vascular dz
- Shrinking lung syndrome

26

The most common pleuropulmonary manifestation of lupus by a long shot is:

Pleuritis (present in 20% at onset, 50% over time, and 100% at autopsy)

27

Lupus pleuritis is typically

Fibrinous

28

Is fibrinous pleuritis specific for lupus>

Nope.

29

Pleural effusions in lupus are typically

small and bilateral

30

Acute lupus pneumonitis

rare, actually a form of acute lung injury, can take form of diffuse hemorrhage